Pulmonary Disease in Progressive Systemic Sclerosis

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Abstract
• Thirteen patients with progressive systemic sclerosis were studied to evaluate the possible role of gastroesophageal reflux as a contributing pathogenic factor in the pulmonary disease of the patients. The evaluation of all patients included fiberoptic esophagogastroduodenoscopy with biopsies of the esophagus, otolaryngologic evaluation, technetium Tc 99m sulfur colloid aspiration scan, pulmonary function testing, including the diffusing capacity for carbon monoxide (DLCO) test, and 24-hour intraesophageal pH monitoring with probes placed 5 and 15 cm above the lower esophageal sphincter. Eleven patients had microscopic and macroscopic evidence of proximal esophagitis, 12 patients had laryngeal changes suggestive of aspiration, and 12 patients had abnormal DLCO values. Using multiple regression analysis, the degree of DLCO impairment correlated with the proximal and distal reflux episodes and scores recorded by pH monitoring. There was direct and indirect evidence for proximal gastroesophageal reflux and aspiration in the majority of patients, and a distinct correlation between the severity of reflux and the severity of pulmonary disease. Aggressive antireflux therapy may be helpful in reducing the pulmonary damage due to aspiration in these patients. (Arch Intern Med1989;149:589-593)